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Original authors: Rapha Onyeka, Stephanie Trejo Corona, Jeff Huynh, Lucy Mudie, Michael T. Yen

Disease Entity

Disease

Vasculitis is an inflammatory condition that impacts blood vessels, disrupting blood flow and potentially leading to tissue damage, with etiologies ranging from autoimmune disorders to infections and drug reactions.1 Orbital vasculitis specifically affects the blood vessels in the orbit, the area around the eyes, and can result in serious vision problems. Inflammation in this region restricts blood supply, causing ischemia and necrosis, which can quickly lead to compromised vision. Due to the rich vascular supply in the orbit, orbital vasculitis often presents with early symptoms, making it a critical sign of underlying, potentially life-threatening systemic vasculitis. Clinicians have the opportunity to intervene with medical and/or surgical approaches and improve patient outcomes if detected early.

Clinical / Histological Classifications

The International Chapel Hill Consensus Conference on the Nomenclature of Systemic Vasculitides was first held in 1994 to name, define, and categorize systemic vasculitides by producing a nomenclature system, which was later revised in 2012.2 Inflammation of the blood vessel walls due to noninfectious causes were categorized based on the size of the vessels predominantly involved: large-, medium-, and small-vessel vasculitis. When vasculitis presents with no predominant vessel size involved, the vasculitis is categorized as variable vessel vasculitis (VVV).

Large-vessel vasculitides include Takayasu arteritis and giant cell arteritis (GCA). The large-vessels affected include the aorta and its major branches. Both large-vessel vasculitides share the same granulomatous histopathologic features. Medium-vessel vasculitides include polyarteritis nodosa (PAN) and Kawasaki Disease. The medium-vessels affected are the main visceral arteries and their branches. Small-vessel vasculitides include three anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and four immune complex small-vessel vasculitis (SVV). The small-vessels affected are the small intraparenchymal arteries, arterioles, capillaries, and venules.

The ANCA-associated vasculitides and immune complex SVV are differentiated by the amount of immune deposits in vessel walls, with the latter presenting with more immune deposition. The ANCA-AAV include microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), and eosinophilic granulomatosis with polyangiitis (EGPA) (formerly known as Churg-Strauss syndrome). Single-organ AAV may also fall into this category if the vasculitis distribution occurs within the small vessels of a single organ, such as renal-limited AAV. Necrotizing inflammation is present in MPA, whereas necrotizing granulomatous inflammation is present in GPA and EGPA. More specifically, EGPA histology is characterized by eosinophilic infiltration, necrotizing vasculitis, and extravascular granulomas. Immune complex SVV includes anti-glomerular basement membrane (anti-GBM) disease, cryoglobulinemic vasculitis (CV), IgA vasculitis (IgAV) (formerly known as Henoch-Schonlein purpura), and hypocomplementemic urticarial vasculitis (HUV) (anti-C1q vasculitis). Vasculitis associated with probable etiologies or vasculitis associated with systemic disease may also fall into this category. Variable vessel vasculitides include Behçet's disease and Cogan syndrome. Cogan syndrome is a necrotizing vasculitis that can involve the aorta, renal arteries and coronary arteries.3 Connective tissue diseases, such as systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA), and inflammatory myopathies, such as dermatomyositis, can result in vascular inflammation as well.4

Etiology

Orbital vasculitides can arise from a variety of etiologies, including autoimmune, inflammatory, and idiopathic conditions.4 Autoimmune conditions such as GPA, RA, GCA, and SLE present in the orbit when the immune system targets blood vessels, leading to inflammation. Behçet's disease, EGPA, and polyarteritis nodosa are classified as systemic vasculitides that often present with orbital involvement. Other inflammatory conditions such as sarcoidosis, Cogan syndrome, and Kawasaki disease have orbital complications. Additionally, dermatomyositis, while primarily affecting muscles and skin, may occasionally manifest with orbital vasculitis. These varied etiologies highlight the importance of systemic evaluation in patients presenting with vasculitis within the orbit, as the initial differential diagnoses may be broad.

Pathophysiology

Systemic vasculitides have complex clinical presentations in various organs, and vessels of the eye may present at any point in different parts of the eye such as the conjunctiva, episclera, sclera, uvea, cornea, and retina.5 The underlying immunopathogenesis of vasculitides relies on the onset of inflammatory reactions via one of the four hypersensitivity reactions (Table 1). Type I is the anaphylactic type, which includes EGPA; type II is the cytotoxic type, which includes Kawasaki disease, MPA, and GPA; type III is the immune-complex type, which includes PAN, IgAV, CV, and vasculitis as a result of SLE, RA, and dermatomyositis; and type IV is the cell-mediated type, which includes GCA, Takayasu arteritis, and GPA.

Type I hypersensitivity reactions involve elevated IgE levels and eosinophilia, leading to vessel inflammation and tissue damage. Type II hypersensitivity reactions involve antibodies that bind to antigens in the vessels as seen in ANCA-AAV. ANCA-activated neutrophils induce endothelial damage and undergo apoptosis, which leads to inflammation and vessel damage. Type III hypersensitivity reactions involve immune complex formation and vessel deposition, which leads to complement activation and neutrophil attraction. This ultimately results in vessel damage via release of lysosomal enzymes. Type IV hypersensitivity reactions involve T-cell-mediated responses that result in granuloma formation. The manifestation of ocular symptoms results from eosinophilic infiltration, granuloma formation, immune complex deposition, and ultimately inflammatory damage to the vessels in various parts of the eye.

Table 1: Hypersensitivity reaction types and their corresponding vasculitides
Type I Anaphylactic Type	Type II Cytotoxic Type	Type III Immune-complex Type	Type IV Cell-mediated type
Eosinophilic granulomatosis with polyangiitis	Granulomatosis with polyangiitis	Polyarteritis nodosa	Giant cell arteritis
	Microscopic polyangiitis	IgA vasculitis	Takayasu arteritis
	Kawasaki disease	Cryoglobulinemic vasculitis	Granulomatosis with polyangiitis

Diagnosis

Anatomic Presentations

The clinical presentation of orbital vasculitis varies widely and is nonspecific, involving orbital structures such as orbital fat and/or nerves, extraocular muscles, lacrimal glands, and the retrobulbar optic nerve.4 The orbital manifestations of vasculitides broadly include ocular pain, vision loss, eyelid erythema, eyelid edema, diplopia, proptosis, afferent pupillary defect, and extraocular muscle movement limitations.4 The clinical entities associated with orbital vasculitis are GCA, PAN, GPA, EGPA, Kawasaki disease, Behçet's disease, atypical Cogan syndrome, SLE/RA, and dermatomyositis. The ocular manifestations of vasculitides include but are not limited to scleritis, episcleritis, uveitis, keratitis, and retinal vasculitis. As a general overview, scleritis and episcleritis may be present in GCA, PAN, GPA, EGPA, IgAV; conjunctivitis may be present in PAN, Kawasaki disease; uveitis may be present in GCA, Takayasu arteritis, PAN, and EGPA; keratitis may be present in Kawasaki disease and IgAV; and retinal vasculitis may be present in Takayasu arteritis, PAN, GPA, MPA, and CV.

Etiological Presentations

In general, most vasculitides may present with constitutional symptoms, such as fever, fatigue, malaise, and/or weight loss. These vasculitides include GCA, Takayasu arteritis, PAN, MPA, GPA, and EGPA.

Giant Cell Arteritis (GCA)

GCA is typically characterized by unilateral headache, polymyalgia rheumatica, jaw and/or arm claudication; and diagnosis is critical to avoid permanent vision loss. Diplopia is a common visual symptom, and further damage to the eye may result in optic neuropathy, central retinal artery occlusion, choroidal ischemia, and uveitis. Patients older than 50 years are the common demographic for GCA. Orbital manifestations include orbital infarction syndrome, ophthalmoplegia, diplopia, vision loss, orbital inflammation, proptosis, chemosis, and conjunctival injection, and afferent pupillary defect.4

Takayasu arteritis

Takayasu arteritis patients may be asymptomatic initially but upper extremity blood pressure discrepancies prompt further evaluation. Visual disturbances (35%) and transient blindness (8%) may progress to neovascularization of the retina or iris.6,7

Polyarteritis Nodosa (PAN)

PAN most commonly presents with mononeuritis multiplex,8 yet other symptoms include livedo reticularis and diastolic blood pressure greater than 90 mmHg.9–11 Orbital involvement of PAN presents as conjunctival injection, ophthalmoplegia, and exophthalmos with symptoms of diplopia, vision loss, orbital inflammation, and chemosis.4

Microscopic Polyangiitis (MPA)

MPA is characterized by chronic constitutional symptoms followed by an explosive phase in which glomerular nephropathy leads to renal deterioration.12 Hemoptysis and dyspnea may also arise if lung involvement is present. Ocular manifestations include peripheral ulcerative keratitis as in PAN.11

Granulomatosis with Polyangiitis (GPA)

GPA usually presents as a classic triad of necrotizing vasculitis involving the respiratory tract, glomerulus, and small arteries and veins.13 Other common features include saddle nose deformity, bloody nasal discharge, cutaneous lesions, ocular involvement, and orbital involvement. Orbital involvement of GPA presents as conjunctival injection, proptosis, and ophthalmoplegia with symptoms of diplopia, vision loss, orbital inflammation, dacryoadenitis, and pain.14

Eosinophilic Granulomatosis with Polyangiitis (EGPA)

EGPA characteristically includes asthma and allergic rhinitis as cardinal features followed by vasculitis that presents approximately three years later with symptoms of abdominal pain, cardiac or renal complications, peripheral neuropathy, and subcutaneous nodules. Ocular manifestations are rare and have been delineated in case reports, including but not limited to diplopia, proptosis, ulcerative conjunctivitis, and peripheral ulcerative keratitis.13 Orbital involvement of EGPA presents as orbital inflammation, dacryoadenitis, proptosis, ophthalmoplegia, episcleritis, and perineuritis with symptoms of diplopia and vision loss.15

Kawasaki disease and IgA Vasculitis (IgAV)

Kawasaki disease and IgAV are unique in that these vasculitides primarily affect children. Kawasaki disease has a constellation of symptoms, including conjunctivitis, skin desquamation, cervical lymphadenopathy, strawberry tongue, erythema and/or edema of the hands and feet, and a fever for at least five days. Besides the characteristic feature of conjunctivitis, dacryocystitis has also been reported.16 Orbital manifestations that have previously been reported include orbital inflammation, unilateral lid edema, and impaired upgaze.17 IgAV also most commonly affects children but can also be seen in adults. Gastrointestinal symptoms such as abdominal pain, diarrhea, and vomiting are common, as are hematuria, arthritis, and palpable purpura on the lower extremities. Ocular manifestations such as scleritis and sclerokeratitis are rare.18

Behçet's disease

Behçet's disease presentation consists of aphthous ulcers, genital ulcers, skin lesions, eye inflammation, or a pathergy reaction.19 Ocular involvement may include uveitis or retinal vasculitis, and orbital involvement includes extraocular muscle inflammation and dacryoadenitis.4

Atypical Cogan syndrome

Atypical Cogan syndrome occurs when Cogan syndrome is accompanied by ocular manifestations and rarely manifests with orbital involvement. The syndrome presents with bilateral nonsyphilitic interstitial keratitis and involves the audiovestibular system, with ocular manifestations, such as blurry vision, conjunctival injection, eye pain, scleritis, retinitis, and artery occlusion, as well as exophthalmos and orbital inflammation.4

Systemic Lupus Erythematosus (SLE) / Rheumatoid Arthritis (RA)

Systemic lupus erythematosus and rheumatoid arthritis present with polyneuropathy and cutaneous lesions, retinopathy, keratoconjunctivitis sicca, and uveitis.4,20 Orbital manifestations are more prevalent in SLE than RA and include orbital inflammation, orbital infarction, myositis, exophthalmos, and limited extraocular muscle movements.4,21

Rheumatoid arthritis associated cutaneous lesions. Image courtesy of Michael T. Yen, MD

Dermatomyositis

Dermatomyositis classically presents with proximal muscle weakness, and case reports have reported extraocular involvement, bilateral exophthalmos, and orbital myositis.21

Clinical Presentation

When evaluating a patient for possible orbital vasculitis, gathering clinically useful information is essential. Vasculitides can present with unique constellations of symptoms that affect multiple organ systems; additionally, vasculitides may share overlapping features. Key initial details to collect include the character of the symptoms, the time of onset, and whether the symptoms affect one or both eyes (monocular or binocular). Common systemic symptoms reported during history-taking include fever, headache, weakness, and malaise.5 In contrast, more specific historical findings can involve neurological dysfunction, ocular/orbital disturbances, nasopharyngeal disease, renal disease, respiratory difficulties, gastrointestinal discomfort, and cardiac involvement.5

Ophthalmic manifestations are most common in giant cell arteritis, Takayasu arteritis, GPA, Behçet’s disease, Cogan syndrome, rheumatoid arthritis, and sarcoidosis; however, ophthalmic manifestations have been reported in most forms of vasculitis.1

Inflammatory lesions from vasculitis can lead to scleritis, keratitis, uveitis, optic neuropathy, and optic neuritis with some lesions being more suggestive of specific vasculitides.22 For instance, optic neuropathy and vision loss are common symptoms of giant cell arteritis, whereas symptoms of interstitial keratitis (like pain, photophobia, and conjunctival injection) alongside vestibulo-auditory dysfunction are characteristic of Cogan syndrome.22 Generally, these eye manifestations cause impaired vision, pain, and visible ocular inflammation on inspection.22 In addition to visual defects, erythema and edema of the eyelids, diplopia, proptosis, ptosis, afferent pupillary defect, and extraocular dysfunction may be more suggestive of orbital involvement.15 Orbital findings are notably prevalent in patients with GPA due to compressive soft tissue swelling.15 Findings from a thorough history should ultimately serve as the basis for subsequent evaluation and diagnostic workup.

Clinical diagnosis

When evaluating a patient for possible orbital vasculitis, gathering clinically useful information is essential. Key details to collect include the nature of the symptoms, such as erythema, tenderness, and visual disturbances. It is also important to determine the time of onset and whether the symptoms affect one or both eyes (monocular or binocular).

Previous episodes of similar symptoms should be noted, along with any history of infections, recent immunizations, or new medications. A thorough personal and family history of autoimmune or systemic inflammatory diseases is critical. Additional factors to consider are recent travel, exposure to sick contacts, substance use, and any relevant environmental exposures. Collecting this information helps in forming a comprehensive differential diagnosis for orbital vasculitis.14

On examination, the pupils should be checked for a relative afferent pupillary defect, ocular motility should be examined for any restriction, and any proptosis should be measured. Eyelid edema and erythema, conjunctival injection or chemosis and tenderness, or enlargement of the lacrimal gland should be noted. Associated systemic signs such as a saddle-nose deformity in GPA or malar/heliotrope rashes should also be looked for on examination.15

Diagnostic procedures

For GPA, orbital disease is confirmed by an orbital biopsy, which typically shows vasculitis, granulomatous inflammation, and/or necrosis in 75-85% of cases. Imaging such as CT and MRI can help distinguish GPA from other conditions with characteristic findings like hyper-intense lesions on CT and hypo-intense lesions on MRI that enhance with gadolinium.

In EGPA, biopsy of affected orbital structures, such as the extraocular muscles or lacrimal glands, is key to diagnosis with necrotizing vasculitis and eosinophilic infiltration being histological markers. While CT and MRI may show nonspecific enlargement of the retro-orbital space and swelling of orbital structures, systemic findings help confirm the diagnosis.

For PAN, diagnosis is more challenging as there are no well-defined imaging or histological markers. Orbital inflammation combined with systemic signs of PAN is diagnostic, and while CT and MRI findings are limited, orbital biopsies may reveal vasculitis with fibrosis; this is not always reliable, however.

GCA can be diagnosed through a combination of symptoms, laboratory tests, and temporal artery biopsy with imaging showing orbital contrast enhancement or mass lesions. Biopsy results may reveal giant cells, artery wall changes, and fibrosis. In rare cases, orbital biopsy can also confirm GCA.

In Behcet’s disease, CT and MRI may show enhancement and enlargement of the extraocular muscles and lacrimal glands. For dermatomyositis, MRI can show enlargement of specific eye muscles, and electromyography may suggest myopathy. However, biopsy of affected muscles is the definitive diagnostic tool.

For Kawasaki disease, CT can identify orbital involvement, and biopsy may show edematous orbital fat and panarteritis, though findings are less specific.

For SLE, an orbital CT may show enlargement of one or more extraocular muscles, which may help inform diagnosis in conjunction with systemic findings. Tissue biopsy may also be pursued to rule out similar-presenting etiologies.

Other notable conditions (RA, sarcoidosis, Cogan syndrome, Takayasu, MPA) do not have many documented cases or diagnostic procedures performed; these conditions are primarily diagnosed from systemic findings and laboratory markers.

Laboratory tests

Vasculitis is associated with numerous inflammatory markers and laboratory findings depending on the etiology. Initial laboratory tests include complete blood count with differential, serum chemistry panel, urinalysis, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) levels, and thyroid-stimulating hormone and free thyroxine serum testing.5 Additional testing may also be done to rule out infectious causes of a patient’s presenting symptoms, such as HIV, syphilis, and tuberculosis.1 Following initial tests, the diagnostic workup may then proceed with more specific testing measures, such as antibody testing.

Table 2. Vasculitis etiologies and corresponding serum markers / laboratory findings

Vasculitis Etiology	Elevated Serum Markers and/or Laboratory Studies
Granulomatosis with polyangiitis (Wegener’s Granulomatosis)	c-ANCA, thrombocytosis, leukocytosis, ESR, CRP; anemia; proteinuria, hematuria
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss Syndrome)	p-ANCA, IgE, eosinophilia
Microscopic polyangiitis	p-ANCA
IgA vasculitis (Henoch-Schonlein Purpura)	IgA; hematuria, proteinuria
Cryoglobulinemic vasculitis	Cryoglobulinemia; decreased C4 levels
Kawasaki disease	ESR, CRP, thrombocytosis, alpha1-antitrypsin
Polyarteritis nodosa	ESR, CRP, eosinophilia, anti-nuclear antibody, rheumatoid factor, HBV/HCV studies; hematuria, proteinuria
Giant cell arteritis	ESR, CRP, HLA-DRB1, fibrinogen, anticardiolipin antibodies
Takayasu arteritis	ESR, CRP
Behçet’s disease	HLA-B51, ESR, CRP, neutrophilia
Cogan syndrome	Anti-heat shock protein 70 antibody
Systemic Lupus Erythematosus	anti-dsDNA antibody, anti-Smith antibody, anti-nuclear antibody, antiphospholipid antibodies; leukopenia, thrombocytopenia, anemia, decreased C3 and C4 levels
Rheumatoid Arthritis	anti-CCP, ESR, CRP, thrombocytosis, leukocytosis, rheumatoid factor; anemia
Dermatomyositis	CK, ESR, CRP, leukocytosis
Sarcoidosis	Hypercalcemia, ESR, CRP, angiotensin-converting-enzyme; leukopenia

Certain laboratory findings are common across several types of vasculitides. Due to the chronic inflammatory processes of vasculitides, ESR and CRP are common but nonspecific findings in most patients. Anemia of chronic disease also arises as a consequence of chronic inflammation. Other potential tests include serum complement tests to evaluate for complement-consuming immune complex vasculitides, such as in mixed cryoglobulinemia and systemic lupus erythematosus (SLE).5 Anti-neutrophilic cytoplasmic antibodies (ANCA) are strongly associated with vasculitides as well, particularly GPA, EGPA, and microscopic polyangiitis; however, ANCA can be positive in other inflammatory and/or autoimmune conditions, like ulcerative colitis, rheumatoid arthritis, and SLE.5 Consequently, markers alone are rarely diagnostic of vasculitides, and a broad differential must be maintained despite certain laboratory findings.5

Large- and medium-vessel vasculitides, including giant cell arteritis, Takayasu arteritis, Kawasaki disease, and polyarteritis nodosa, generally present with nonspecific findings of elevated ESR and CRP. More unique findings have been found in patients with giant cell arteritis, which has been linked to HLA-DRB1, elevated serum fibrinogen, and positive anticardiolipin antibodies.1, 5 Polyarteritis nodosa can occur in association with hepatitis B infection and therefore often presents with hepatitis B positive serology.15

ANCA is believed to be related to the pathogenesis of certain vasculitides, resulting in a class of ANCA-associated vasculitides that are often positive for the antibodies c-ANCA or p-ANCA. These antibodies target neutrophils expressing cell surface molecules proteinase-3 and myeloperoxidase, respectively.5 In GPA, c-ANCA can appear in up to 80-90% of cases with a sensitivity of 91% and specificity of 99%.15 Other nonspecific findings include anemia, thrombocytosis, and leukocytosis.15 Due to possible renal involvement, patients may present with hematuria and proteinuria on urinalysis, though these findings are also present in polyarteritis nodosa and IgA vasculitis.5 Furthermore, significant cytokine staining for IL-17 and IL-23 have been reported in these patients.5 In contrast, EGPA and microscopic polyangiitis are strongly associated with p-ANCA.15 Due to its association with allergic angiitis, EGPA presents characteristically with elevated eosinophil levels.15

HLA associations have been studied in certain etiologies, including Behçet’s disease and giant cell arteritis, but these associations can vary. For instance, the strength of the association between Behçet’s disease and HLA-B51 is relatively strong in Eastern Europe and Asia but falls in Western countries.1

Other laboratory findings to note are: anti-heat shock protein 70 antibody in Cogan syndrome; rheumatoid factor and anti-cyclic citrullinated protein antibody in rheumatoid arthritis; anti-nuclear antibody, anti-dsDNA antibody, and anti-Smith antibody in SLE; elevated angiotensin-converting enzyme and serum calcium in sarcoidosis; and elevated creatine kinase in dermatomyositis.15

Differential diagnosis

Since vasculitides can present systemically with varied signs and symptoms, there is a wide range of potential diagnoses to consider. The differential diagnosis for orbital vasculitis with systemic involvement encompasses broad categories, including autoimmune disorders, neoplasms, infections, and drug-induced vasculitis.5 Examples of autoimmune disorders include, but are not limited to, sarcoidosis, inflammatory bowel disease, Sjogren’s syndrome, systemic lupus erythematosus, rheumatoid arthritis, and scleroderma - all of which can induce inflammation of the orbit.1 Examples of neoplastic causes are myeloproliferative or lymphoproliferative disorders and lymphoma.1 Bacterial, viral, parasitic, and fungal agents should be considered as possible etiologies.1 Furthermore, exogenous factors such as opioids, antihypertensives, antibiotics, and antithyroid medications, have been linked to orbital vasculitis and may be classified as secondary vasculitides.5 Orbital vasculitis may also present without systemic involvement, such as retinal vasculitis, pars planitis, idiopathic retinal vasculitis aneurysms and neuroretinitis syndrome, and Eales disease.1 Additionally, many forms of non-vasculitic orbital inflammation, notably idiopathic orbital inflammatory disease, can mimic orbital vasculitis and should likewise be considered in the differential diagnosis, especially in cases of unilateral acute orbital pain without prior history of orbital-related systemic disease.1

Management

Medical therapy

The pharmacological treatment of ocular vasculitis varies by the underlying causes. The standard approach for non-infectious orbital vasculitis is immunosuppressive therapy, with corticosteroids being the primary medical intervention. Based on the severity and extent of the disease, steroid-sparing agents may be required for disease control or long-term management. Non-steroidal treatments include methotrexate, azathioprine, and mycophenolate for long-term management. Cases with a higher degree of burden may require rituximab, etanercept, infliximab, and adalimumab, which are biologics with efficacy in controlling inflammation and preventing relapse.23

Table 3. Pharmacotherapies often considered in treatment of vasculitis

Drug	Common Vasculitis Uses	Targets	Adverse Effects
Rituximab	Granulomatosis with Polyangiitis (GPA), Microscopic Polyangiitis (MPA), and Cryoglobulinemic vasculitis	Binds CD20 on B-cells, leading to their depletion	Infusion reactions, infections (especially hepatitis B reactivation), neutropenia, and risk of progressive multifocal leukoencephalopathy (PML)
Etanercept	Rarely used in vasculitis (more common in rheumatoid arthritis, but occasionally in systemic inflammatory diseases like Behçet's disease)	TNF-alpha inhibitor	Injection site reactions, increased risk of infections, demyelinating disease, and possible malignancies
Infliximab	Behçet’s disease and occasionally in other refractory vasculitides	TNF-alpha inhibitor	Increased risk of infections (including TB reactivation), infusion reactions, lupus-like syndrome, and hepatotoxicity
Adalimumab	Behçet’s disease, used off-label in some cases of systemic vasculitis	TNF-alpha inhibitor	Similar to infliximab: infections, TB reactivation, injection site reactions, and risk of malignancy

Surgical Interventions

For orbital masses, particularly common in granulomatosis with polyangiitis (GPA), granulomatous masses often remain challenging to treat, with many cases being refractory to corticosteroid and immunomodulators.24 This can lead to visual impairment and even vision loss in a significant portion of patients. Surgery, such as orbitotomy with orbital decompression or debulking, may be required when symptoms persist. Proper differentiation between active inflammatory masses and fibrotic changes is crucial, as fibrotic masses respond poorly to both medical and surgical treatments.

Non-vision-threatening inflammation (e.g., conjunctivitis or episcleritis) and dry eye syndrome in vasculitis are typically managed non-surgically. Although for cases of epiphora, caused by nasolacrimal duct obstruction leading to excessive tearing in GPA, dacryocystorhinostomy with silicone tubing is the preferred surgical technique if the local inflammation is well-managed24.

References

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